Abstract
Background: As one of the 4 United Kingdom national centres for Lysosomal Storage Disorders, Birmingham Children’s Hospital (BCH) cares for 49 patients with a mucopolysaccharide (MPS) disorder. Presently, their oral health needs have never been fully investigated. Enzyme therapies are revolutionising medical treatment, and will prolong life expectancy. Therefore, oral health and its impact on quality of life are increasingly important.
Hypothesis: Patients with an MPS disorder have greater oral health needs in comparison with the general population.
Design: Forty-six patients with an MPS disorder were investigated for caries, gingival health and enamel opacities. Questionnaires to establish the utilisation of dental services and assess the perceived difficulties in achieving adequate oral health care were completed.
Results: Patients with an MPS disorder have specific oral health needs depending on the type of MPS experienced. This study found 76% of patients with MPS IV had experienced dental caries and they all showed evidence of a generalised unspecified enamel defect. Subjects with MPS I, II and III did not have an increased caries rate; 50% of the study group had received dental treatment in the past, of which 74% (17, n = 23) required a general anaesthetic.
Conclusion: The MPS disorders can have a significant effect on the developing dentition. Subjects with MPS IV do have an increased oral health need in comparison with the general population and the other MPS groups due to their increased caries rate and enamel defects. Further research is required to fully assess their needs and investigate any structural tooth defects.
Competing interests: None declared.
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References
Alpoz AR, Coker M, Celen E, Ersin NK, Gokcen D, Van Diggelenc OP, Huijmansc JG (2006) The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 101:632–7
Bascd (2005) Core protocol for the conduct of epidemiological surveys of 5 year old children in the West Midlands. British Association for the Study of Community Dentistry. 2005/06 protocol. West Midlands PCT ed. West Midlands Primary Care Trust
Gardner DG (1971) The oral manifestations of Hurler's syndrome. Oral Surg Oral Med Oral Pathol, 32:46–57
Guven G, Cehreli ZC, Altun C, Sencimen M, Ide S, Bayari SH, Karacay S (2008) Mucopolysaccharidosis type I (Hurler syndrome): oral and radiographic findings and ultrastructural/chemical features of enamel and dentin. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 105:72–78
Hopkins R, Watson JA, Jones JH, Walker M (1973) Two cases of Hunter's syndrome – the anaesthetic and operative difficulties in oral surgery. Br J Oral Surg, 10:286–99
Keith O, Scully C, Weidmann GM (1990) Orofacial features of Scheie (Hurler-Scheie) syndrome (α-L-iduronidase deficiency). Oral Surg Oral Med Oral Pathol, 70:70–74
Kinirons MJ, Nelson J (1990) Dental findings in mucopolysaccharidosis type IV A (Morquio's disease type A). Oral Surg Oral Med Oral Pathol, 70:176–179
Kircher S, Bajboui M, Miebach E, Beck M (2007) Mucopolysaccharidoses – a guide for physicians and parents. UNI-MED Verlag AG, International Medical Publishers, London, Boston, Printed in Europe
Kumar P, Clark M (1998) Clinical medicine, 4th edn. W.B. Saunders, Harcourt Brace and Company Limited, Italy
Kuratani T, Miyawaki S, Murakami T, Takano-Yamamoto T (2005) Early orthodontic treatment and long-term observation in a patient with Morquio syndrome. Angle Orthod, 75:881–887
Levin LS, Jorgenson RJ, Salinas CF (1975) Oral findings in the Morquio syndrome (mucopolysaccharidosis IV). Oral Surg Oral Med Oral Pathol, 39:390–395
Lewis CW (2009) Dental care and children with special health care needs: a population-based perspective. Acad Pediatr 9:420–426
Lustmann J (1978) Dentinoenamel junction area in primary teeth affected by Morquio's syndrome. J Dent Res 57:475–479
Mcgovern E, Owens L, Nunn J, Bolas AO, Meara A, & Fleming P (2008) Oral findings in patients with Hurler syndrome post stem cell transplantation. International Journal of Paediatric dentistry, 18 (Suppl. 1): 1
Oncag G, Ertan Erdinc AM, Cal E (2006) Multidisciplinary treatment approach of Morquio syndrome (Mucopolysaccharidosis Type IVA). Angle Orthod, 76:335–340
Pitts N, Harker R (2005) Obvious decay experience. Children's Dental Health Survey in the United Kingdom 2003. London, Office for National Statistics
Purohit BM, Acharya S, Bhat M (2010) Oral health status and treatment needs of children attending special schools in South India: a comparative study. Spec Care Dentist 30:235–241
Roberts MW, Barton NW, Constantopoulos G, Butler DP, Donahue AH (1984) Occurrence of multiple dentigerous cysts in a patient with the Maroteaux-Lamy syndrome (mucopolysaccharidosis, type VI). Oral Surg Oral Med Oral Pathol, 58:169–175
Rolling I, Clausen N, Nyvad B, Sindet-Pedersen S (1999) Dental findings in three siblings with Morquio's syndrome. Int J Paediatr Dent, 9:219–224
Santos RP, Hoo JJ (2006) Difficulty in recognizing multiple sulfatase deficiency in an infant. Pediatrics 117:955–958
Schmidt H, Ullrich K, Von Lengerke HJ, Kleine M, Bramswig J (1987) Radiological findings in patients with mucopolysaccharidosis I H/S (Hurler-Scheie syndrome). Pediatr Radiol, 17:409–414
Sela M, Eidelman E, Yatziv S (1975) Oral manifestations of Morquio's syndrome. Oral Surg Oral Med Oral Pathol 39:583–589
Shaw L, Maclaurin ET, Foster TD (1986) Dental study of handicapped children attending special schools in Birmingham, UK. Community Dent Oral Epidemiol 14:24–27
Sheiham A (2005) Oral health, general health and quality of life. Bull World Health Organ 83:644
Smith KS, Hallett KB, Hall RK, Wardrop RW, Firth N (1995) Mucopolysaccharidosis: MPS VI and associated delayed tooth eruption. Int J Oral Maxillofac Surg, 24:176–180
Soong BW, Casamassima AC, Fink JK, Constantopoulos G, Horwitz AL (1988) Multiple sulfatase deficiency. Neurology 38:1273–1275
Thomas S, Tandon S (2000) Hurler syndrome: a case report. J Clin Pediatr Dent 24:335–338
Webman MS, Hirsch SA, Webman H, Stanley HR (1977) Obliterated pulp cavities in the Sanfilippo syndrome (mucopolysaccharidosis III). Oral Surg Oral Med Oral Pathol, 43:734–738
White D, Lader D (2004) Office for National Statistics, Periodontal condition, oral health behaviour and attitudes to oral health. Children's Dental Health Survey in the United Kingdom, 2003. London
Wraith JE (1995) The mucopolysaccharidoses: a clinical review and guide to management. Arch Dis Child 72:263–267
Wraith JE, Scarpa M, Beck M, Bodamer OA, De Meirleir L, Guffon N, Meldgaard Lund A, Malm G, Van der Ploeg AT, Zeman J (2008) Mucopolysaccharidosis type II (Hunter syndrome): a clinical review and recommendations for treatment in the era of enzyme replacement therapy. Eur J Pediatr 167:267–277
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Communicated by: Robin Lachmann.
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Synopsis: MPS disease does have an impact on oral health and the developing dentition, the extent of which varying between disease type. To date the effect of enzyme therapy on the oral health is largely unknown.
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James, A., Hendriksz, C.J., Addison, O. (2011). The Oral Health Needs of Children, Adolescents and Young Adults Affected by a Mucopolysaccharide Disorder. In: JIMD Reports - Case and Research Reports, 2011/2. JIMD Reports, vol 2. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2011_46
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DOI: https://doi.org/10.1007/8904_2011_46
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